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The main biological fluids used are urine, plasma and, in special situations, cerebrospinal fluid. In contrast to commonly used methods such as gas chromatography and high performance liquid chromatography mass spectrometry, ex vivo proton spectroscopy (<jats:sup>1<\/jats:sup>H\u2010NMR) is not yet used in routine clinical practice, although it has been recommended for more than 30\u2009years. Automatic analysis and improved NMR technology have also expanded the applications used for the diagnosis of inborn errors of metabolism. We provide a mini\u2010overview of typical applications, especially in urine but also in plasma, used to diagnose common but also rare genetic metabolic diseases with <jats:sup>1<\/jats:sup>H\u2010NMR. The use of computer\u2010assisted diagnostic suggestions can facilitate interpretation of the profiles. In a proof of principle, to date, 182 reports of 59 different diseases and 500 reports of healthy children are stored. The percentage of correct automatic diagnoses was 74%. Using the same <jats:sup>1<\/jats:sup>H\u2010NMR profile\u2010targeted analysis, it is possible to apply an untargeted approach that distinguishes profile differences from healthy individuals. Thus, additional conditions such as lysosomal storage diseases or drug interferences are detectable. Furthermore, because <jats:sup>1<\/jats:sup>H\u2010NMR is highly reproducible and can detect a variety of different substance categories, the metabolomic approach is suitable for monitoring patient treatment and revealing additional factors such as nutrition and microbiome metabolism. Besides the progress in analytical techniques, a multiomics approach is most effective to combine metabolomics with, for example, whole exome sequencing, to also diagnose patients with nondetectable metabolic abnormalities in biological fluids. In this mini review we also provide our own data to demonstrate the role of NMR in a multiomics platform in the field of inborn errors of metabolism.<\/jats:p>","DOI":"10.1002\/nbm.4853","type":"journal-article","created":{"date-parts":[[2022,10,20]],"date-time":"2022-10-20T11:08:50Z","timestamp":1666264130000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Ex vivo proton spectroscopy (<sup>1<\/sup>H\u2010NMR) analysis of inborn errors of metabolism: Automatic and computer\u2010assisted analyses"],"prefix":"10.1002","volume":"36","author":[{"given":"Claire","family":"Cannet","sequence":"first","affiliation":[{"name":"Bruker Biospin  Ettlingen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Georg","family":"Frauendienst\u2010Egger","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Reutlingen Klinikum Reutlingen, School of Medicine, University of Tuebingen  Reutlingen 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